Assess the determinants of quality of life (QOL) and caregiver burden (CGB) in patients with primary lateral sclerosis (PLS).  

QOL and CGB measurements are increasingly used to guide clinical care of patients with ALS, however it is unclear if research on QOL and CGB in patients with ALS can be applied to patients with PLS since to date no studies to date have investigated QOL or CGB in patients with PLS.

12 self-selected patients with PLS and their respective caregivers/family members were included: 7 women (58%); age: 68.0 [61.50,74.00]; highest education: 1 (8%) high school, 4 (33%) Bachelors, 7 (58%) Masters/PhD; Race/ethnicity: 9 white/Caucasian (75%), 1 Asian/Indian (8%), 1 Indigenous American (8%), 1 Indigenous American & African American (8%); region of onset 10 lower limbs (83%), 2 bulbar (17%). Outcomes: ALS Cognitive-Behavioral Screen, ALS Functional Rating Scale-Revised, McGill Quality of Life Questionnaire, Caregiver Burden Scale, Geriatric Depression Scale, and ALS/PLS-specific Wellness Survey. Statistical analyses: descriptive statistics, bivariate analysis for continuous variables, and Kruskal-Wallis ANOVA for categorical variables. 

Significant positive association shown between functional status and quality of life (r=0.71, p-value=0.01). Cognitive status, behavioral status and depressive symptoms were not significantly associated with quality of life. Social support was most frequently rated as having the largest positive impact on QOL (83%). 33% of participants attend a patient support group. No significant associations with caregiver burden were identified.

This study provides evidence that the clinical care of patients with PLS should prioritize maximizing functional status. This is consistent with studies in patients with ALS. This study also provides evidence for the positive impact of social support for patients with PLS, indicating that further options for social support including PLS specific support groups would likely be beneficial.